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Sickle Cell Anemia
Sickle-Cell Anemia, or Sickle-Cell disease is a genetic disorder in which there is a mutation in Beta Globin. Red blood cells assume an abnormal "C" shape, or sickle shape, instead of the normal round shape. It occurs worldwide but is most common in Africa and Tropic Areas. Currently, it is estimated that it occurs in 1 in every 500 births in Africa and 1 in every 1,500 births in Hispanic Countries. Almost 2 million Africans carry the trait for Sickle-Cell Anemia. Symptoms and Diagnosis Symptoms appear soon (typically 4 months) after birth. One of the first signs is Hand-foot syndrome (or dactylitis) where there is a swelling of the hands or feet. Anemia, or a lack of red blood cells, is a common symptom of the disorder. Anemia may result in dizziness, headaches, fatigue, and difficulty in breathing. Most patients experience episodes of pain that can last anywhere from a few hours to a few days. The frequency of these episodes is random, but usually they occur once every few years. They occur mainly in the abdominal, chest. back, and long bone areas. Those with Sickle-Cell Anemia are at a higher risk of having infections which can result in frequent fevers and Aplastic Crisis (decrease in Red Blood Cell production by bone marrow). They are also have a higher risk of having a stroke. Prognosis Typically, those with Sickle-Cell Anemia die between the ages of 20-30 years old. With modern progress in healthcare, there has been a higher frequency in those with Sick-Cell Anemia who live 50 years or more. The most common cause of death is organ failure. Inheritance A person can only inherit Sickle-Cell Anemia if both parents carry the gene. When a person inherits one normal gene and one gene for the disorder, it is called "Sickle Cell Trait," or Sicklemia. It is an example of Codominance Inheritance. These people are usually normal, though they may show some minor symptoms of the disorder. Studies have shown that Sicklemia results in a higher resistance to Malaria. Thus, it can be explained why Sickle Cell Trait is very common in Africa (estimated 25% of the population has it) and other areas where Malaria is common. One form of Thalassemia, b-Thalassemia, causes a lack of normal, health hemoglobin. Thalassemia Trait, or Thalassemia Minor, occurs when a person inherits one gene for Sickle-Cell Anemia and one gene for b-Thalassemia. The result can be just as bad as Sickle-Cell Anemia. This occurrence can happen with another form of Thalassemia called Thalassemia C. Treatment Currently, the most effective treatment is a Bone Marrow Transplant though it is only done of the most severe cases as they are very risky. Blood transfusions are usually done to lower the risk of stroke. Patients are suggested to take folic acid as it promotes red blood cell production. Hydroxyurea is sometimes taken to reduce pain episodes, but it is not effective on everyone. With a higher risk of infection, patients need to be properly vaccinated and take antibiotics when needed. Celebrities with Sickle Cell Anemia Paul Williams, Singer (The Temptations) Tiki Barber T-Boz Larenz Tate, Actor Miles Davis, Jazz Musician Georgeanna Tillman, Singer (The Marvelettes) Prodigy, Rapper (Mobb Deep) Sources http://www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_WhatIs.html http://www.ncbi.nlm.nih.gov/omim/603903 http://www.nlm.nih.gov/medlineplus/ency/article/000527.htm http://kidshealth.org/parent/medical/heart/sickle_cell_anemia.html http://sickle.bwh.harvard.edu/scd_inheritance.html http://www.iesee.org/health/football-superstar-tiki-barber-says-be-sickle-smart.html